Pemphigus vulgaris is an uncommon, debilitating, potentially fatal skin disorder characterized by intraepidermal bullae that appear on normal appearing skin without surrounding inflammation.
Pemphigus is associated with HLA-A10 and HLA-DR4 antigens. There is a higher incidence among persons of Jewish or Mediterranean descent.
Pemphigus is an autoimmune disorder with specific IgG antibodies and sometimes complement which are deposited at sites of epidermal cell damage. Many medications (particularly penicillamine) can cause pemphigus.
Pemphigus may be an adverse reaction to thiol groups found in most drugs and the Allium family, which includes garlic, onions and leeks.
Serum IgG and IgA gliadin antibodies have been found to be increased in patients with pemphigus. This may be due to increased intestinal permeability . Alternatively gliadin may somehow precipitate the autoimmune process.
Dysfunctional calcium metabolism may also be present in pemphigus. The pemphigus foliaceus antigen appears to contain a calcium-sensitive epitope, and in pemphigus vulgaris, alteration in the function of calcium-sensitive cadherins may play a role in the production of acantholysis.
Pemphigus may have a hormonal component. Pemphigus is also associated with pregnancy, and studies have shown increased levels of estradiol and reduced ones of testosterone. Lowered DHEA levels have also been found. DHEA deficiency is often found in autoimmune diseases, and may contribute to their etiology and/or pathophysiology. Increased levels of ACTH and hydrocortisone have been found in patients with pemphigus.
Autoantibody titers (by immunofluorescent studies) are routinely ordered. Titer corresponds to severity of the disease.
Causative antigens are located on the exterior surface of the cytoplasmic membrane of epithelial cells. Biopsy shows acantholytic intraepidermal bullae. Light microscopy shows suprabasal cleft formation and acantholysis (tumor formed by proliferation of epithelial squamous cells).
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